Intravascular leiomyomatosis is a rare, histologically benign disease, and can spread through the venous system to the right cavities of the heart. We report the case of a 39-year-old woman who presented with intravenous leiomyomatosis with extension to the right ventricle, causing heart failure. She underwent surgery successfully, undergoing a total resection of this tumor through the infrarenal cava and the hysterectomy in one surgical stage. Surgical technique considerations are discussed and the literature is reviewed.